Chronic glucocorticoid management in neuromuscular disease: A survey of neuromuscular neurologists.

INTRODUCTION/AIMS: Glucocorticoids (GC) are first-line therapy for many neuromuscular diseases. There is a lack of guidelines regarding the prevention and management of GC complications in the context of neuromuscular disease, introducing the potential for practice variation, that may compromise quality of care. Our aim was to evaluate the practice patterns among Canadian adult neuromuscular neurologists on the screening, management, and treatment of GC-related complications and to identify variances in practice. METHODS: A web-based anonymous questionnaire was disseminated to 99 Canadian adult neuromuscular neurologists. Questions addressed patterns of screening, prevention, monitoring, and treatment of GC-induced adverse events, including infection prophylaxis, vaccination, bone health, hyperglycemia, and other complications. RESULTS: Seventy-one percent completed the survey. Of those, 52% perform screening blood work prior to initiating GC, 56% screen for infections, and 18% for osteoporosis. The majority monitor glycemic control and blood pressure (>85%). Thirty-two (46%) reported that they do not primarily monitor GC complications, but rather provide recommendations to the primary care physician. Pneumocystis jiroveci pneumonia prophylaxis was never used by 29%, and 29% recommend vaccinations prior to GC initiation. Calcium supplementation was recommended by 80% to prevent osteoporosis. Only 36% were aware of any existing guidelines for preventing GC complications, and 91% endorsed a need for neurology-specific guidelines. DISCUSSION: There is substantial variability in the management of GC adverse effects among neuromuscular neurologists, often not corresponding to limited published literature. Our results support the need for improved education and neurology-specific guidelines to help standardize practice and improve and prevent complications.

Pain perception during electrodiagnostic studies and the impact of learners.

INTRODUCTION/AIMS: Electrodiagnostic testing (EDX) may be perceived as painful, which may influence patient expectations and test completion. Our aim was to characterize which component of the EDX was more painful, and to determine if any factors, particularly performance by a learner, influenced this perception. METHODS: Participants were prospectively recruited and completed a brief questionnaire to rate their perception of pain before and after each component of the EDX. Demographic information and information about the test itself was collected. RESULTS: A total of 251 participants were recruited, 55.3% female, with a mean age of 52.9 y. Most participants rated pain after nerve conduction studies (NCS) and needle electromyography (EMG) as mild to moderate with a similar number rating each component as more painful than the other. There was no effect of sex on overall ratings, although females felt that the test was more painful than anticipated more often than males. If a learner performed the EMG, it was more likely that the test would be rated as moderately to severely painful, and more likely for the EMG to be rated as more painful than the NCS (p < .05). DISCUSSION: The finding that NCS and EMG perceived pain were similar may help accurately inform patients of test expectations, guide test planning and help reduce the likelihood of incomplete or canceled testing. More effort might be required to help mitigate EMG pain when learners are involved.

Patient preference for virtual versus in-person visits in neuromuscular clinical practice.

INTRODUCTION/AIMS: It is unknown if patients with neuromuscular diseases prefer in-person or virtual telemedicine visits. We studied patient opinions and preference on virtual versus in-person visits, and the factors influencing such preferences. METHODS: Telephone surveys, consisting of 11 questions, of patients from 10 neuromuscular centers were completed. RESULTS: Five hundred and twenty surveys were completed. Twenty-six percent of respondents preferred virtual visits, while 50% preferred in-person visits. Sixty-four percent reported physical interaction as "very important." For receiving a new diagnosis, 55% preferred in-person vs 35% reporting no preference. Forty percent were concerned about a lack of physical examination vs 20% who were concerned about evaluating vital signs. Eighty four percent reported virtual visits were sufficiently private. Sixty eight percent did not consider expenses a factor in their preference. Although 92% were comfortable with virtual communication technology, 55% preferred video communications, and 19% preferred phone calls. Visit preference was not significantly associated with gender, diagnosis, disease severity, or symptom management. Patients who were concerned about a lack of physical exam or assessment of vitals had significantly higher odds of selecting in-person visits than no preference. DISCUSSION: Although neither technology, privacy, nor finance burdened patients in our study, more patients preferred in-person visits than virtual visits and 40% were concerned about a lack of physical examination. Interactions that occur with in-person encounters had high importance for patients, reflecting differences in the perception of the patient-physician relationship between virtual and in-person visits.

Practical guidance for managing electromyography requests and testing during the COVID-19 pandemic.

The COVID-19 pandemic has necessitated cancelation of elective or nonurgent contact with the healthcare system, including nonurgent nerve conduction studies and electromyography (electrodiagnostic [EDX] studies). The definitions of elective and nonurgent are physician judgments, and often are not straightforward. Clinical care must be provided to help our patients in a timely manner, while keeping them, healthcare personnel, and the community safe. Benefit/risk stratification is an important part of this process. We have stratified EDX studies into three categories: Urgent, Non-urgent, and Possibly Urgent, in an effort to help clinicians triage these referrals. For each category, we provide a rationale and some examples. However, each referral must be reviewed on a case-by-case basis, and the clinical situation will evolve over time, necessitating flexibility in managing EDX triaging. Engaging the referring clinician and, at times, the patient, may be useful in the triage process.

Guidance for resumption of routine electrodiagnostic testing during the COVID-19 pandemic.

As the world accommodates to the coronavirus disease 2019 (COVID-19) pandemic, routine in-person medical services are resuming. The resumption of non urgent electrodiagnostic (EDX) testing faces unique challenges due to the long duration of the procedure and direct close contact with patients, including studies with risk of exposure to oropharyngeal secretions. We provide consensus guidance for resumption of EDX testing, addressing scheduling, patient arrival and registration, use of personal protective equipment, COVID-19 screening and testing, the performance of EDX testing in outpatient and inpatient settings, cleaning and maintenance of the EDX equipment and laboratory, balancing trainee safety and training requirements, and patient care issues. These are broad recommendations that need to be adapted to local COVID-19 risks, institutional guidelines and policies, and changing federal, state, and local regulations, and to changes in the pandemic over time.

Delayed Thrombectomy Center Arrival is Associated with Decreased Treatment Probability.

BACKGROUND: Endovascular thrombectomy (EVT) is effective in reducing disability in selected patients with stroke and large vessel occlusion (LVO), but access to this treatment is suboptimal. AIM: We examined the proportion of patients with LVO who did not receive EVT, the reasons for non-treatment, and the association between time from onset and probability of treatment. METHODS: We conducted a retrospective cohort study of consecutive patients with acute stroke and LVO presenting between January 2017 and June 2018. We used multivariable log-binomial models to determine the association between time and probability of treatment with and without adjustment for age, sex, dementia, active cancer, baseline disability, stroke severity, and evidence of ischemia on computerized tomography. RESULTS: We identified 256 patients (51% female, median age 74 [interquartile range, IQR 63.5, 82.5]), of whom 59% did not receive EVT. The main reasons for not treating with EVT were related to occlusion characteristics or infarct size. The median time from onset to EVT center arrival was longer among non-treated patients (218 minutes [142, 302]) than those who were treated (180 minutes [104, 265], p = 0.03). Among patients presenting within 6 hours of onset, the relative risk (RR) of receiving EVT decreased by 3% with every 10-minute delay in arrival to EVT center (adjusted RR 0.97 CI95 [0.95, 0.99]). This association was not found in the overall cohort. CONCLUSIONS: The proportion of patients with acute stroke and confirmed LVO who do not undergo EVT is substantial. Minimizing delays in arrival to EVT center may optimize the delivery of this treatment.

The Virtual Neurologic Exam: Instructional Videos and Guidance for the COVID-19 Era.

OBJECTIVE: To outline features of the neurologic examination that can be performed virtually through telemedicine platforms (the virtual neurological examination [VNE]), and provide guidance for rapidly pivoting in-person clinical assessments to virtual visits during the COVID-19 pandemic and beyond. METHODS: The full neurologic examination is described with attention to components that can be performed virtually. RESULTS: A screening VNE is outlined that can be performed on a wide variety of patients, along with detailed descriptions of virtual examination maneuvers for specific scenarios (cognitive testing, neuromuscular and movement disorder examinations). CONCLUSIONS: During the COVID-19 pandemic, rapid adoption of virtual medicine will be critical to provide ongoing and timely neurological care. Familiarity and mastery of a VNE will be critical for neurologists, and this article outlines a practical approach to implementation.

Musculocutaneous neuropathy.

INTRODUCTION: Isolated musculocutaneous neuropathy is uncommon. In this study we aimed to determine its causes and clinical presentation and interpret the electrodiagnostic findings associated with this condition. METHODS: Our investigation was a retrospective review of patients diagnosed with musculocutaneous neuropathy at the Mayo Clinic (Rochester, Minnesota) electromyography (EMG) laboratory between 1997 and 2015. RESULTS: Thirty-two patients with musculocutaneous neuropathy and 5 patients with lateral antebrachial cutaneous neuropathy were identified. The most common cause was acute trauma or surgery (65%). Fourteen percent of the cases were idiopathic and 14% were inflammatory. Pain and sensory disturbance were more common presentations than weakness. Weakness from nerve injury was not noted in 2 patients, suggesting that other muscles may provide adequate elbow flexion/supination. The bilateral absence of lateral antebrachial cutaneous nerve sensory responses suggests an inflammatory cause. DISCUSSION: Musculocutaneous neuropathy usually results from trauma or iatrogenic injury. Nerve conduction studies alone are insufficient to confirm neuropathy, and needle EMG examination should be a routine part of the diagnostic evaluation. Muscle Nerve 58: 726-729, 2018.

In-Patient Code Stroke: A Quality Improvement Strategy to Overcome Knowledge-to-Action Gaps in Response Time.

BACKGROUND AND PURPOSE: Stroke is a relatively common and challenging condition in hospitalized patients. Previous studies have shown delays in recognition and assessment of inpatient strokes leading to poor outcomes. The goal of this quality improvement initiative was to evaluate an in-hospital code stroke algorithm and educational program aimed at reducing the response times for inpatient stroke. METHODS: An inpatient code stroke algorithm was developed, and an educational intervention was implemented over 5 months. Data were recorded and compared between the 36-month period before and the 15-month period after the intervention was implemented. Outcome measures included time from last seen normal to initial assessment and from last seen normal to brain imaging. RESULTS: During the study period, there were 218 inpatient strokes (131 before the intervention and 87 after the intervention). Inpatient strokes were more common on cardiovascular wards (45% of cases) and occurred mainly during the perioperative period (60% of cases). After implementation of an inpatient code stroke intervention and educational initiative, there were consistent reductions in all timed outcome measures (median time to initial assessment fell from 600 [109-1460] to 160 [35-630] minutes and time to computed tomographic scan fell from 925 [213-1965] to 348.5 [128-1587] minutes). CONCLUSIONS: Our study reveals the efficacy of an inpatient code stroke algorithm and educational intervention directed at nurses and allied health personnel to optimize the prompt management of inpatient strokes. Prompt assessment may lead to faster stroke interventions, which are associated with better outcomes.

Detecting fasciculations in cranial nerve innervated muscles with ultrasound in amyotrophic lateral sclerosis.

INTRODUCTION: Cranial muscle fasciculations may be difficult to detect in amyotrophic lateral sclerosis (ALS). Ultrasound (US) detection of fasciculations in these muscles may have clinical utility. METHODS: Patients with suspected ALS were prospectively enrolled. Nerve conduction studies, needle electromyography (EMG), and US examination of cranial muscles were performed. Controls were examined by US only. Fasciculations were counted and scored for each muscle after 10 or 30 seconds. RESULTS: There were 84 patients with ALS. Fasciculations were most frequently found in the genioglossus muscle. Overall, detection rates by US and EMG were similar, but US was more likely to detect frequent fasciculations. Fasciculations were rare in controls, seen in 7 of 1,090 (0.6%) muscles. No control had > 5 fasciculations in any muscle. DISCUSSION: Fasciculations were frequently detected in cranial muscles of patients with ALS. US was found to be a sensitive method, and was not impaired by factors such as anxiety and the inability of the patient to relax. Muscle Nerve 56: 1072-1076, 2017.

The risk of iatrogenic pneumothorax after electromyography.

INTRODUCTION: Pneumothorax is a potentially serious complication of electromyography (EMG). Data on the frequency of pneumothorax after EMG are lacking. The purpose of this study was to determine the frequency, timing, and risk factors for iatrogenic pneumothorax after EMG. METHODS: Cases of pneumothorax after EMG were reviewed for clinical, electrophysiological, and radiological data. RESULTS: Of 64,490 EMG studies, 7 patients had an association between the EMG and pneumothorax. All patients were symptomatic and presented within 24 hours of EMG. Sampling of serratus anterior and diaphragm was causative in 1 patient each. In 5 patients, multiple high-risk muscles were sampled. The highest frequency of pneumothorax was observed with examination of serratus anterior (0.445%) and diaphragm (0.149%). CONCLUSIONS: The frequency of symptomatic iatrogenic pneumothorax after EMG appears to be low, and examinations of serratus anterior and diaphragm carry the highest risk. Electromyographers should be aware of the risk of pneumothorax and should counsel patients accordingly.

Adult polyglucosan body disease presenting as a unilateral progressive plexopathy.

INTRODUCTION: Adult polyglucosan body disease (APBD) usually presents with progressive spastic paraparesis, neurogenic bladder, and distal lower limb sensory abnormalities. It is caused by mutations in the glycogen branching enzyme gene (GBE1). METHODS: We describe a woman with an unusual phenotype manifesting as progressive left brachial more than lumbosacral plexopathies, with central sensory and corticospinal tract involvement. RESULTS: Magnetic resonance imaging of the brain and cervical spine showed abnormal T2 signal within the ventral pons and medulla bilaterally, involving the pyramidal tracts and the medial leminisci. There was also medullary and cervical spine atrophy. On nerve biopsy, large polyglucosan bodies were noted in the endoneurium. The patient was found to be compound heterozygous for 2 novel mutations in GBE1. Peripheral blood leukocyte GBE activity was markedly reduced to 7% of normal, confirming the diagnosis of APBD. CONCLUSIONS: In this report we describe a new phenotype of APBD associated with 2 novel mutations. Muscle Nerve 53: 976-981, 2016.

The utility of genetic testing in neuromuscular disease: A consensus statement from the AANEM on the clinical utility of genetic testing in diagnosis of neuromuscular disease.

INTRODUCTION: The aim of this consensus statement is to provide a recommendation from AANEM experts on the clinical utility of genetic testing. It is not meant to recommend or endorse any specific genetic testing methodology or algorithm. METHODS: The AANEM Professional Practice Committee reached a consensus based on expert opinion on the utility of genetic testing in neuromuscular diseases and made recommendations on factors that physicians and patients should consider when deciding whether to proceed with such testing. RESULTS: Despite the costs of genetic testing, these tests can be both valuable and beneficial in the diagnosis and treatment of neuromuscular diseases in many situations. CONCLUSIONS: The AANEM believes that performing genetic testing to arrive at a specific molecular diagnosis is a critical step in providing high-quality care to neuromuscular patients. The cost of testing should not be a deterrent, as there are important clinical, safety, psychosocial, and research benefits. Muscle Nerve 54: 1007-1009, 2016.

Electromyographic findings in 37 patients with adult-onset acid maltase deficiency.

INTRODUCTION: In acid maltase deficiency (AMD), electrical myotonia (EM) may be restricted to paraspinal muscles. A comprehensive description of the electromyographic (EMG) findings in AMD is lacking. The purpose of this study is to describe the EMG features in adult-onset AMD, focusing on the distribution of EM. METHODS: A retrospective chart review of AMD patients diagnosed at Mayo Clinic over age 18 years. RESULTS: Thirty-seven patients were included. Twenty-eight (76%) had EM in at least 1 muscle, and EM was more common in paraspinal and proximal limb muscles. The tensor fasciae latae (TFL) was equally sensitive to the paraspinals for EM. Three of 4 patients had EM identified in the diaphragm. CONCLUSIONS: Approximately three-quarters of adult-onset AMD patients display EM on EMG. The paraspinal muscles and TFL are the most likely to demonstrate EM, and EM can be detected in the diaphragm of adult onset AMD patients.

Effects of napping on neuromuscular fatigue in myasthenia gravis.

INTRODUCTION: The relationship between sleep and neuromuscular fatigue is understood poorly. The goal of this study was to evaluate the effects of napping on quantitative measures of neuromuscular fatigue in patients with myasthenia gravis (MG). METHODS: Eight patients with mild to moderate MG were recruited. Patients underwent maintenance of wakefulness tests (MWT) and multiple sleep latency tests (MSLT). The Quantitative Myasthenia Gravis Score (QMGS) was measured before nap and after each nap to examine the effects of napping and sleep on neuromuscular weakness. RESULTS: Results showed that QMGS improves only after naps where patients slept more than 5 min but not where patients did not sleep or slept less than 5 min. CONCLUSIONS: Daytime napping mitigates neuromuscular fatigue in patients with MG, especially if patients slept for more than 5 min.